System Maintenance Notice

Please note that maintenance work is scheduled between 4 AM and 5 AM - CET. During this time, you may experience reduced performance. Thanks for your patience.

Publications

Journal Article Review Epublish

PubMed: 40701643 DOI: 10.1183/16000617.0046-2025

Treating connective tissue disease-associated interstitial lung disease - think outside the box: a perspective.

Eur Respir Rev 34(177)

Authors: Yet H Khor, Catharina C Moor, Wolfgang Merkt, Katharina Buschulte, Boyang Zheng, Veronika Müller, Michael Kreuter

Affiliations

  • Respiratory Research@Alfred, Central Clinical School, Monash University, Melbourne, Australia. Department of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Australia. Institute for Breathing and Sleep, Heidelberg, Australia. Faculty of Medicine, University of Melbourne, Melbourne, Australia.
  • Department of Respiratory Medicine, Erasmus Medical Center, Rotterdam, The Netherlands.
  • Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany.
  • Department of Hematology, Oncology and Rheumatology, Internal Medicine V, University Hospital Heidelberg, Heidelberg, Germany.
  • Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL), Heidelberg, Germany.
  • Department of Medicine, University of British Columbia, Vancouver, BC, Canada.
  • Department of Pulmonology, Semmelweis University, Budapest, Hungary.
  • Mainz Center for Pulmonary Medicine, Department of Pneumology, Mainz University Medical Center and Department of Pulmonary, Critical Care and Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany.

Abstract

Connective tissue disease-associated interstitial lung disease is one of the most common subtypes of interstitial lung disease, which is a leading cause of morbidity and mortality in patients with these systemic autoimmune rheumatic diseases. A spectrum of disease trajectories exists within individual and across different connective tissue diseases. In individuals with connective tissue diseases who are at risk or at the early asymptomatic stage with interstitial lung changes, we have potential windows of opportunity for interventions to prevent the development of or progression to interstitial lung disease. In this perspective, we use systemic sclerosis and rheumatoid arthritis as sample cases to discuss emerging knowledge on disease pathogenesis, as well as to apply the preventative medicine concept for pharmacotherapeutic approaches at different disease stages of connective tissue disease-associated interstitial lung disease.

MeSH

Lung, Connective Tissue, Connective Tissue Diseases, Lung Diseases, Tissues, Lung Diseases, Interstitial